The Vascular Reconstruction for Post-Kasai Biliary Atresia Splenic Malformation in Living Donor Liver Transplantation.

Abstract

Aims: Biliary atresia (BA) is a major indication for liver transplantation in infants. These infants have a high frequency of associated malformations known as biliary atresia splenic malformation (BASM) syndrome. Anatomical abnormalities in patients with BA often include polysplenia, preduodenal portal vein, interrupted retrohepatic inferior vena cava, cardiac abnormalities and situs inversus. In living donor liver transplant (LDLT) patients who had congenital vascular anomalies that compounded the technical hazards of the operation, additional surgical modifications for the vascular reconstruction are sometimes necessary to achieve satisfactory liver perfusion. We aimed to evaluate outcomes of patients with BASM syndrome. Methods: A retrospective analysis was performed on medical records of LDLT patients. Patients with a diagnosis of BA were identified. Results: Between 1991 and 2013, 82 LDLTs for post-Kasai BA were performed at Tohoku University Hospital. There were 49 female (59.8%) and 33 male (40.2%) pediatric recipients. Mean age at transplant was 1.1±9.6 years (5 months-44 years), with 50 children (61%) being less than 2 years of age at LDLT. The overall 1-, 5-, and 10-year patient survival in these 82 LDLT patients with BA was 90.2%, 89.0%, and 87.4%, respectively. Of these, 3 patients (3.7%) had BASM syndrome: absence of retrohepatic vena cava (3), intestinal malrotation (2), preduodenal portal vein (1), hepatic artery anomalies (0), cardiac anomalies (1), and situs inversus (1). LDLTs were performed at the patient age of 8 months to 10 years. A right liver lobe without middle hepatic vein was used in 1 case and a left liver lobe with MHV was used in 2 cases. A superficial femoral vein as a vascular graft was obtained from living donor and used for middle and inferior hepatic veins reconstruction in 1 case. In a left liver graft, we created single larger orifice using recipient's LHV, MHV, and RHV, and then reconstructed the LHV and MHV of the donor graft directly to this orifice without vascular conduits. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 57 months. Conclusions: LDLT can be successfully performed in pediatric patients with BASM syndrome. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications.